井上 淑子¹, 斎藤 聡², 伝法 秀幸¹, 山口 和磨¹, 窪田 幸一¹, 田矢 祐規³, 和氣 敦³, 増田 亜希子¹, 石綿 一哉³

Yoshiko INOUE¹, Satoshi SAITOH², Hideyuki DENPO¹, Kazuma YAMAGUCHI¹, Koichi KUBOTA¹, Yuki TAYA³, Atsushi WAKE³, Akiko MASUDA¹, Kazuya ISHIWATA³

¹虎の門病院分院臨床検査部, ²虎の門病院肝臓センター, ³虎の門病院分院血液内科

¹Department of Clinical Laboratory, Toranomon Hospital Kajigaya, ²Department of Hepatology, Toranomon Hospital, ³Department of Hematology, Toranomon Hospital Kajigaya

キーワード : liver stiffness, transient elastography, hematopoietic stem cell transplantation (HSCT), sinusoidal obstruction syndrome (SOS), veno-occlusive disease (VOD)

症例は32歳女性，急性骨髄性白血病の治療のため同種造血幹細胞移植が施行された．入院時の肝硬度（liver stiffness: LS）は3.5 KPaであった．移植後，第67病日から体重増加，腹痛，血清ビリルビン値の上昇を認め，腹部超音波検査（Ultrasonography: US），肝硬度測定を実施した．LSは42.2 KPaと著明に上昇，US検査では肝腫大，腹水貯留，門脈血流低下を認め，遅発性の肝類洞閉塞症候群／中心静脈閉塞症（sinusoidal obstruction syndrome/veno-occlusive disease: SOS/VOD）と診断された．遺伝子組み換えトロンボモジュリン，新鮮凍結血漿，低分子ヘパリン投与後，肝硬度低下とともに門脈血流は改善，血清ビリルビン値も減少した．LSは第114病日25.4 KPa，第342病日には4.9 KPaに低下した．SOS/VODの診断は主に臨床症状からなされ，欧州血液骨髄移植学会による遅発性SOS/VOD診断基準（2016年）では肝血行動態を含むUS所見が記載された．しかしながらドプラ血流測定には検者の熟練と時間を要する．肝硬度は検者を選ばず，短時間に，簡便に，繰り返し測定が可能である．遅発性SOS/VODの診断，経過観察に肝硬度測定の有用性が示唆された．

A 32-year-old woman had undergone allogeneic hematopoietic stem cell transplantation for the treatment of acute myeloid leukemia. Liver stiffness (LS) at admission was 3.5 KPa. Weight gain, abdominal pain, and elevated serum total bilirubin level were observed approximately 67 days after transplantation. Ultrasonography (US) and LS measurement (LSM) were performed. LS was markedly high (at 42.2 KPa), and US revealed hepatomegaly, ascites, and decreased portal venous flow. The patient was diagnosed with late-onset sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD). After administration of recombinant thrombomodulin, fresh frozen plasma, and low-molecular-weight heparin, LS decreased, portal blood flow improved, and serum bilirubin level decreased. Moreover, LS decreased to 25.4 and 4.9 KPa after 115 and 342 days, respectively. The diagnosis of SOS/VOD was based on clinical symptoms and US findings, and the hepatic hemodynamics were included in the diagnostic criteria for late-onset SOS/VOD (European Society for Blood marrow transplantation, 2016). However, Doppler blood flow measurement is skill-dependent and time-consuming. LS can be measured quickly, easily, and repeatedly, even by unskilled examiners. Therefore, LSM may be useful for the diagnosis and follow-up of late-onset SOS/VOD.